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Titolo:
Diagnosis, treatment and outcome of patients with Askin-tumors
Autore:
Christiansen, S; Semik, M; Dockhorn-Dworniczak, B; Rotker, J; Thomas, M; Schmidt, C; Jurgens, H; Winkelmann, W; Scheld, HH;
Indirizzi:
Univ Munster, Klin & Poliklin Thorax Herz & Gefasschirurg, D-48149 Munster, Germany Univ Munster Munster Germany D-48149 sschirurg, D-48149 Munster, Germany Univ Munster, Gerhard Domagk Inst Pathol, D-48149 Munster, Germany Univ Munster Munster Germany D-48149 st Pathol, D-48149 Munster, Germany Univ Munster, Klin & Poliklin Hamatol Onkol, D-48149 Munster, Germany UnivMunster Munster Germany D-48149 tol Onkol, D-48149 Munster, Germany Univ Munster, Klin & Poliklin Anasthesiol & Operat Intens Med, D-48149 Munster, Germany Univ Munster Munster Germany D-48149 ntens Med, D-48149 Munster, Germany Univ Munster, Klin & Poliklin Padiat Hamatol Onkol, D-48149 Munster, Germany Univ Munster Munster Germany D-48149 tol Onkol, D-48149 Munster, Germany Univ Munster, Orthopad Klin & Poliklin, D-48149 Munster, Germany Univ Munster Munster Germany D-48149 Poliklin, D-48149 Munster, Germany
Titolo Testata:
THORACIC AND CARDIOVASCULAR SURGEON
fascicolo: 5, volume: 48, anno: 2000,
pagine: 311 - 315
SICI:
0171-6425(200010)48:5<311:DTAOOP>2.0.ZU;2-#
Fonte:
ISI
Lingua:
ENG
Soggetto:
PERIPHERAL NEUROECTODERMAL TUMORS; MOLECULAR-GENETIC DETECTION; THORACOPULMONARY REGION; CELL TUMOR; CHILDHOOD; SURVIVAL; ENTITY;
Keywords:
Askin tumor; primitive neuroectodermal tumor; multimodality therapy;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Citazioni:
17
Recensione:
Indirizzi per estratti:
Indirizzo: Christiansen, S Univ Munster, Klin & Poliklin Thorax Herz & Gefasschirurg,Albert Schweitzer Str 33, D-48149 Munster, Germany Univ Munster Albert Schweitzer Str 33 Munster Germany D-48149
Citazione:
S. Christiansen et al., "Diagnosis, treatment and outcome of patients with Askin-tumors", THOR CARD S, 48(5), 2000, pp. 311-315

Abstract

Askin tumors are highly malignant small-round-cell tumors of the thoracopulmonary region, which occur rarely. Therefore, we report on our experienceswith eight patients (5 male, 3 females). who were treated in our department between 11'94 and 10'97 (age: 9-40 years, mean age: 20.5 years). All Askin tumors were diagnosed by histological and immunohistochemical examinations as well as moleculargenetic proof of characteristic translocations. In all patients, the tumor arose from the chest wall, infiltrating adjacent ribsand parts of the lung. At the time of first diagnosis, five patients did not reveal any metastases. One patient suffered from intrapulmonary metastases and two patients from an infiltration of the diaphragm and of adjacent vertebral bodies. Treatment consisted of a pre- and postoperative (radio-) chemotherapy according to the EVAIA protocol and a radical tumor resection in all patients. The postoperative course was uneventful in seven patients, one patient suffered from pneumonia after multiple wedge resections for intrapulmonary metastases. Four patients, in whom primary tumor resection was complete, are alive 14, 20, 35 and 84 months after first diagnosis - only one patient had to undergo a second operation for a local relapse 17 months after first diagnosis. The other 4 patients, who suffered from a very extensive primary tumor, expired 13, 17, 18 and 39 months after the diagnosis was made. Our data demonstrate that Askin tumors require an aggressive multimodality treatment consisting of pre- and postoperative chemotherapy, radical surgical resection and postoperative irradiation, which may be performed preoperatively in selected cases, too.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 16:47:02